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  • Surgical Pathology Criteria
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    Precursor B Lymphoblastic Lymphoma

    Definition

    • Lymphoma with morphologic and immunologic features of precursor B cells; at presentation the primary site of involvement must not be bone marrow or peripheral blood

    Alternate/Historical Names

    • Lymphoblastic lymphoma, convoluted or non-convoluted

    Diagnostic Criteria

    • Morphologically indistinguishable from B Lymphoblastic Leukemia
      • Presence of a mass lesion and <25% blasts in marrow required for diagnosis of lymphoma
        • Criteria for separation are arbitrary
      • >80% of precursor B neoplasms are leukemic
      • Precursor B leukemia and lymphoma are considered manifestations of the same disease
    • Diffuse effacement of node architecture
      • No true nodular pattern
        • May be pseudonodular with fine fibrous bands
      • Rare partial node involvement
        • Paracortical and parafollicular
      • Frequent soft tissue involvement
      • Fragile cells may show crush or smear artifact
    • Small to medium sized cells in most cases
      • Atypical or large cell variant
        • Reported in 10% of cases
        • Slightly larger nuclei
        • May contain nucleoli
        • No clinical significance
    • Round to oval nuclei with fine chromatin
      • Thin nuclear membrane
      • Nucleoli few and inconspicuous
      • Convoluted nuclear membranes in most cases
      • Frequent mitotic figures
    • Scant cytoplasm
      • Imprints show rare vacuoles and coarse PAS+ granules
    • Immature B phenotype

    Yasodha Natkunam MD PhD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting:: May 1, 2006

    Supplemental studies

    Immunohistology

    • TdT positive
    • HLA-DR positive
    • B lineage positive
      • CD19 positive
      • CD79a cytoplasmic
      • CD22 cytoplasmic
      • Surface immunoglobulin does not preclude the diagnosis
    • CD10 positive
    • Myeloid antigens may infrequently be expressed
      • CD13, CD33, CD15
      • May be associated with t(9;22) or 11q23 abnormalities, which have unfavorable prognosis
      • CD10-/CD15+ phenotype in adults is associated with therapy related leukemia/lymphoma with 11q23 abnormalities, which has unfavorable prognosis
    • Scoring system for lineage assignment of leukemias by the European Group for the Immunologic Classification (EGIL)

      Score

      B-lymphoid

      T-lymphoid

      Myeloid

      2

      Cytoplasmic CD79a*

      CD3 (membrane/cytoplasmic)

      MPO

       

      Cytoplasmic IgM

      Anti-TCR

       

       

      Cytoplasmic CD22

       

       

       

       

       

       

      1

      CD19

      CD2

      CD117

       

      CD20

      CD5

      CD13

       

      CD10

      CD8

      CD33

       

       

      CD10

      CD65

       

       

       

       

      0.5

      TdT

      TdT

      CD14

       

      CD24

      CD7

      CD15

       

       

      CD1a

      CD64

      • A score of 2.5 is considered sufficient to assign a lineage
      • CD79a may also be expressed in a subset of T lymphoblastic leukemia/lymphoma

    Genetic analysis

    Abnormality Gene fusion Frequency Prognosis

    t(9;22)(q34;q11.2)

    BCR/ABL

    3-4%

    Unfavorable

    t(4;11)(q21;q23)

    AF4/MLL

    2-3%

    Unfavorable

    t(1;19)(q23;q13.3)

    PBX/E2A

    6% (25% of pre-B type)

    Unfavorable

    t(12;21)(q13;q22)

    TEL/AML1

    16-29%

    Favorable

    Hyperdiploid chromosomes 4,10,17

     

    20-25%

    Favorable

    Hypodiploid

     

    5%

    Unfavorable

    Modified from Brunning et al. in Jaffe 2001

     

    Cytochemistry

    • Myeloperoxidase negative
    • Sudan Black B negative

     

    Differential Diagnosis

    Precursor B Lymphoblastic Leukemia / Lymphoma Precursor T Lymphoblastic Leukemia / Lymphoma
    10% of precursor lymphoblastic lymphomas 90% of precursor lymphoblastic lymphomas
    Frequently involves skin and bone Frequent mediastinal mass
    B lineage (usually CD19+, CD79a+ at a minimum) T lineage (usually CD3+, CD7+ at a minimum)
    Both are TdT+ and may be CD34+


    Burkitt Lymphoma Lymphoblastic Lymphoma, T and B
    Median age 30 years Median age 17-20 years
    B lineage markers positive 90% T, 10% B
    Multiple nucleoli Inconspicuous nucleoli
    Coarse chromatin Fine chromatin
    Thin rim of cytoplasm Scant cytoplasm
    Frequently GI or ovary T frequently mediastinal, B frequently cutaneous
    TdT negative TdT virtually always positive
    Translocation involving myc gene No myc translocation
    Both have extremely high mitotic rates and starry sky macrophages although Burkitt lymphoma typically has a higher mitotic rate and the starry sky pattern is more uniform


    Lymphoblastic Lymphoma, T or B Blastic Mantle Cell Lymphoma
    Median age 17-20 years Rare under 30 years
    Histiocytes infrequent (tingible body macrophages frequent) Scattered histiocytes frequent
    No prior diagnosis of mantle zone lymphoma Frequent diagnosis of mantle zone lymphoma
    T lineage 90%, B lineage 10% B lineage markers positive
    bcl1 negative bcl1 85%
    CD5 negative if B lineage CD5 80%

     

    Lymphoblastic Lymphoma, B or T Extramedullary Myeloid Tumor
    90% T lineage, 10% B lineage CD43+ but other T and B markers negative
    Myeloperoxidase negative Myeloperoxidase >90%
    Lysozyme negative Lysozyme 90%

     

    Lymphoblastic Lymphoma, B or T DLBCL
    Median age 17-20 years Median age 60 years
    90% T lineage, 10% B lineage B lineage
    Starry sky macrophages present Starry sky pattern infrequent
    Fine chromatin Vesicular chromatin
    None or small nucleoli May have prominent nucleoli
    Uniform cells Heterogeneous cells
    Generally only a problem in suboptimally processed specimens

     

    Lymphoblastic Lymphoma, B or T SLL/CLL
    Median age 17-20 years Rare under 30 years
    Starry sky macrophages frequent Starry sky macrophages rare
    High mitotic rate Mitoic figures only in proliferation centers
    T lineage 90%, B lineage 10% B lineage
    CD23 negative CD23 85%
    Generally only a problem in suboptimally prepared specimens in which cytologic detail is obscured

     

      Rhabdomyosarcoma PPNET Neuroblastoma Lymphoblastic Lymphoma, B or T
    Muscle markers positive negative negative negative
    CD99 positive negative negative positive
    LCA and or B/T lineage negative negative negative 90% T, 10% B
    TdT negative negative negative positive
    Cytoplasm abundant, eosinophilic scant fibrillar scant

     

     

     

    Clinical

    • Median age 20 years
    • Frequently involves lymph nodes, skin, bone
      • Bone marrow and blood may be involved
        • By definition <25% marrow blasts

    Grading / Staging / Report

    • High grade by definition

    Ann Arbor Staging System

    • Stage I
      • I if involvement of a single lymph node region
      • IE if involvement of a single extralymphatic organ or site
    • Stage II
      • II if two or more lymph node regions on same side of diaphragm
      • IIE if localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm
    • Stage III
      • III if Involvement of lymph node regions on both sides of the diphragm
      • IIIS if spleen involved
      • IIIE if extralymphatic site involved
    • Stage IV
      • Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement
    • Systemic Symptoms in 6 months preceding admission
      • Fever, night sweats, 10% weight loss
      • A = absent
      • B = present
    • Extranodal sites are also designated
      • M+ = marrow
      • L+ = lung
      • H+ = liver
      • P+ = pleura
      • O+ = bone
      • D+ = skin and subcutaneous tissue
    • Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also used for non-Hodgkin lymphomas.

    The pathology report should contain the following information:

    • Diagnosis in the World Health Organization (WHO) classification
      • Equivalent diagnosis in other classifications used by relevant clinicians
    • Results of supplementary studies if performed
    • Relationship to other specimens from the same patient
    • Information relevant to staging if available

     

    Lists

    Blastic lymphomas


    Types and variants of B cell lymphoma

    Bibliography

    • Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman R . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
    • Jaffe ES, Harris NL Stein H, Vardiman JW eds. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
    • Carroll WL, Bhojwani D, Min DJ, Raetz E, Relling M, Davies S, Downing JR, Willman CL, Reed JC. Pediatric acute lymphoblastic leukemia. Hematology (Am Soc Hematol Educ Program). 2003;:102-31.
    • Kansal R, Deeb G, Barcos M, Wetzler M, Brecher ML, Block AW, Stewart CC. Precursor B lymphoblastic leukemia with surface light chain immunoglobulin restriction: a report of 15 patients. Am J Clin Pathol. 2004 Apr;121(4):512-25.
    • Ishizawa S, Slovak ML, Popplewell L, Bedell V, Wrede JE, Carter NH, Snyder DS, Arber DA. High frequency of pro-B acute lymphoblastic leukemia in adults with secondary leukemia with 11q23 abnormalities. Leukemia. 2003 Jun;17(6):1091-5.
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